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Orbit alveolar rhabdomyosarcoma

MedGen UID:
235408
•Concept ID:
C1335126
•
Neoplastic Process
Synonyms: Alveolar Rhabdomyosarcoma of Orbit; alveolar rhabdomyosarcoma of orbit; Alveolar Rhabdomyosarcoma of the Orbit; alveolar rhabdomyosarcoma of the orbit; Orbit Alveolar Rhabdomyosarcoma; orbit alveolar rhabdomyosarcoma
 
Monarch Initiative: MONDO:0002978

Definition

A malignant mesenchymal neoplasm that arises in the orbit. It is characterized by the presence of round cells with myoblastic differentiation and a fibrovascular stroma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOrbit alveolar rhabdomyosarcoma

Professional guidelines

PubMed

Adult head and neck rhabdomyosarcoma: radiotherapy- based treatment, outcomes, and predictors of survival.
Zhao D, Zhou F, Liu W, Huang Z, Xu X, Zheng B, Liu C, Bai C, Liu J, Sun Y, Wang W, Xiao S
BMC Cancer 2024 Mar 14;24(1):340. doi: 10.1186/s12885-024-12079-y. PMID: 38486204Free PMC Article
Frequency and Clinical Course of Residual Orbital Masses After Treatment of Orbital Rhabdomyosarcoma.
Sobel RK, Ford JR, Dong W, Shriver E, Griepentrog GJ, Debnam JM, Esmaeli B
Am J Ophthalmol 2022 Feb;234:28-36. Epub 2021 Jul 17 doi: 10.1016/j.ajo.2021.06.035. PMID: 34280364
Pediatric head and neck rhabdomyosarcoma: An analysis of treatment and survival in the United States (1975-2016).
Darwish C, Shim T, Sparks AD, Chillakuru Y, Strum D, Benito DA, Monfared A
Int J Pediatr Otorhinolaryngol 2020 Dec;139:110403. Epub 2020 Sep 25 doi: 10.1016/j.ijporl.2020.110403. PMID: 33049553

Recent clinical studies

Etiology

Rhabdomyosarcoma of the maxillofacial region in children and adolescents: Report of 9 cases and literature review.
Iatrou I, Theologie-Lygidakis N, Schoinohoriti O, Tzermpos F, Vessala AM
J Craniomaxillofac Surg 2017 Jun;45(6):831-838. Epub 2017 Mar 23 doi: 10.1016/j.jcms.2017.03.005. PMID: 28431807
Histology, Fusion Status, and Outcome in Alveolar Rhabdomyosarcoma With Low-Risk Clinical Features: A Report From the Children's Oncology Group.
Arnold MA, Anderson JR, Gastier-Foster JM, Barr FG, Skapek SX, Hawkins DS, Raney RB Jr, Parham DM, Teot LA, Rudzinski ER, Walterhouse DO
Pediatr Blood Cancer 2016 Apr;63(4):634-9. Epub 2016 Jan 12 doi: 10.1002/pbc.25862. PMID: 26756883Free PMC Article
Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data.
Turner JH, Richmon JD
Otolaryngol Head Neck Surg 2011 Dec;145(6):967-73. Epub 2011 Aug 26 doi: 10.1177/0194599811417063. PMID: 21873599
Extensive alveolar-type paranasal sinus and orbit rhabdomyosarcoma with intracranial invasion treated successfully.
Chen SC, Bee YS, Lin MC, Sheu SJ
J Chin Med Assoc 2011 Mar;74(3):140-3. Epub 2011 Feb 19 doi: 10.1016/j.jcma.2011.01.031. PMID: 21421211
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992

Diagnosis

Alveolar rhabdomyosarcoma metastatic to the brain.
Rodriguez-Beato F, De Jesus O, Sanchez-Ortiz J, Delgado P, Perez-Berenguer JL, Labat EJ
BMJ Case Rep 2021 Mar 4;14(3) doi: 10.1136/bcr-2020-240516. PMID: 33664037Free PMC Article
Gene Fusions in Ocular Adnexal Tumors.
Milman T, Ida CM, Zhang PJL, Eagle RC Jr
Am J Ophthalmol 2021 Jan;221:211-225. Epub 2020 Aug 13 doi: 10.1016/j.ajo.2020.08.012. PMID: 32800827
A Review of the Role of Cytogenetics in the Diagnosis of Orbital Rhabdomyosarcoma.
Cortes Barrantes P, Jakobiec FA, Dryja TP
Semin Ophthalmol 2019;34(4):243-251. Epub 2019 May 30 doi: 10.1080/08820538.2019.1620802. PMID: 31146616
Orbital cavitary rhabdomyosarcoma: case report and literature review.
Yazıcı B, Sabur H, Yazıcı Z
Ophthalmic Plast Reconstr Surg 2014 Jan-Feb;30(1):e20-2. doi: 10.1097/IOP.0b013e31828de376. PMID: 23552609
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992

Therapy

Worse Outcomes for Head and Neck Rhabdomyosarcoma Secondary to Reduced-Dose Cyclophosphamide.
Casey DL, Wexler LH, Wolden SL
Int J Radiat Oncol Biol Phys 2019 Apr 1;103(5):1151-1157. Epub 2018 Nov 30 doi: 10.1016/j.ijrobp.2018.11.049. PMID: 30508617Free PMC Article
Head and neck rhabdomyosarcoma in children: a 20-year retrospective study at a tertiary referral center.
Häußler SM, Stromberger C, Olze H, Seifert G, Knopke S, Böttcher A
J Cancer Res Clin Oncol 2018 Feb;144(2):371-379. Epub 2017 Nov 16 doi: 10.1007/s00432-017-2544-x. PMID: 29143871
Alveolar rhabdomyosarcoma with multiple distal metastases. A case report and review of literature.
Sarkar D, Ray S, Saha M, Chakrabarti P
BMJ Case Rep 2012 Sep 3;2012 doi: 10.1136/bcr-2012-006523. PMID: 22948994Free PMC Article
Iodine-125 orbital brachytherapy with a prosthetic implant in situ.
Stannard C, Maree G, Munro R, Lecuona K, Sauerwein W
Strahlenther Onkol 2011 May;187(5):322-7. Epub 2011 Apr 26 doi: 10.1007/s00066-011-2177-y. PMID: 21533761
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992

Prognosis

Alveolar rhabdomyosarcoma metastatic to the brain.
Rodriguez-Beato F, De Jesus O, Sanchez-Ortiz J, Delgado P, Perez-Berenguer JL, Labat EJ
BMJ Case Rep 2021 Mar 4;14(3) doi: 10.1136/bcr-2020-240516. PMID: 33664037Free PMC Article
Gene Fusions in Ocular Adnexal Tumors.
Milman T, Ida CM, Zhang PJL, Eagle RC Jr
Am J Ophthalmol 2021 Jan;221:211-225. Epub 2020 Aug 13 doi: 10.1016/j.ajo.2020.08.012. PMID: 32800827
A Review of the Role of Cytogenetics in the Diagnosis of Orbital Rhabdomyosarcoma.
Cortes Barrantes P, Jakobiec FA, Dryja TP
Semin Ophthalmol 2019;34(4):243-251. Epub 2019 May 30 doi: 10.1080/08820538.2019.1620802. PMID: 31146616
Histology, Fusion Status, and Outcome in Alveolar Rhabdomyosarcoma With Low-Risk Clinical Features: A Report From the Children's Oncology Group.
Arnold MA, Anderson JR, Gastier-Foster JM, Barr FG, Skapek SX, Hawkins DS, Raney RB Jr, Parham DM, Teot LA, Rudzinski ER, Walterhouse DO
Pediatr Blood Cancer 2016 Apr;63(4):634-9. Epub 2016 Jan 12 doi: 10.1002/pbc.25862. PMID: 26756883Free PMC Article
Orbital rhabdomyosarcoma.
Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S
Cancer Control 2004 Sep-Oct;11(5):328-33. doi: 10.1177/107327480401100507. PMID: 15377992

Clinical prediction guides

Adult head and neck rhabdomyosarcoma: radiotherapy- based treatment, outcomes, and predictors of survival.
Zhao D, Zhou F, Liu W, Huang Z, Xu X, Zheng B, Liu C, Bai C, Liu J, Sun Y, Wang W, Xiao S
BMC Cancer 2024 Mar 14;24(1):340. doi: 10.1186/s12885-024-12079-y. PMID: 38486204Free PMC Article
MRI features of different types of sinonasan rhabdomyosarcomas: a series of eleven cases.
Zeng J, Liu L, Li J, Huang Q, Pi L, Jin K
Dentomaxillofac Radiol 2021 Dec 1;50(8):20210030. Epub 2021 Apr 9 doi: 10.1259/dmfr.20210030. PMID: 33835837Free PMC Article
Spectrum of Biopsy Proven Extraocular Muscle Tumours of Non-Thyroid Origin.
Afghani T, Mansoor H, Mughal AM, Asif M, Asif M, Hamdani NR
J Coll Physicians Surg Pak 2019 Jun;29(6):553-557. doi: 10.29271/jcpsp.2019.06.553. PMID: 31133156
An unusual case of rhabdomyosarcoma presenting as orbital apex syndrome.
Machleder DJ, Banik R, Rosenberg RB, Parikh SR
Int J Pediatr Otorhinolaryngol 2005 Feb;69(2):249-54. doi: 10.1016/j.ijporl.2004.07.018. PMID: 15656960
Risk-based management: current concepts of treating malignant solid tumors of childhood.
Grosfeld JL
J Am Coll Surg 1999 Oct;189(4):407-25. doi: 10.1016/s1072-7515(99)00167-2. PMID: 10509467

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